Pseudarthrosis of the radius associated with neurofibromatosis: report of a case and review of the literature

An 11-year-old child with pseudarthrosis of the radius associated with neurofibromatosis was treated by conventional bone graft. Five years after the operation, the pseudarthrosis united, but the grafted bone was slightly sclerotic and bowed. This article describes the clinical findings and postoperative results, and discusses the current surgical approaches in the treatment of pseudarthrosis of the radius.     

Congenital pseudarthrosis of the forearm in a neurofibromatosis patient: case report and review of the literature

We report a case of congenital pseudarthrosis of the forearm associated with neurofibromatosis type 1 which was treated by free vascularized periosteal flap transplant and repeated bone grafting. The young female patient recovered good hand and forearm function. A review of the literature revealed the rare occurrence of this disease (approximately 100 cases reported to date), which probably explains the wide variety of surgical proposals.     

Angiomyolipoma with regional lymph node involvement: a case report and literature review

A 28-year-old man without tuberous sclerosis, who complained of pollakisuria, consulted to our hospital for a left renal mass. Abdominal computed tomography revealed a solid mass without a lipid component, 10 cm in diameter, in the left kidney and with regional lymphadenopathy. Renal arteriography showed a hypervascular mass, demonstrating multiple tumor stains and aneurysms. Left radical nephrectomy and perihilar lymph node dissection were performed for an anticipated diagnosis of malignant tumor in November 2001. The histopathological diagnosis was an angiomyolipoma with lymph node involvement. Immunostaining for myogen markers was positive in the renal mass and lymph node tumors. He was free from disease ten months after surgery.     

Valvular involvement in ANCA-associated systemic vasculitis: a case report and literature review

Background  

Antineutrophil cytoplasmic antibodies (ANCA)-associated systemic vasculitides have a variety of presentations, but cardiac valvular involvement is rarely diagnosed and its management is not established.     

Secondary central nervous system involvement by follicular lymphoma: case report and review of the literature

BACKGROUND: We report a patient with indolent stage IV follicular lymphoma, grade 1, initially successfully treated with chemotherapy, who later developed aggressive diffuse large B-cell lymphoma in the parieto-occipital lobe 8 years after initial presentation. The differing patterns of lymphomatous involvement of the central nervous system (CNS) are briefly reviewed, with a focus on the patterns seen in secondary CNS spread by low-grade lymphomas. CASE DESCRIPTION: A 53-year-old man was diagnosed with stage IV follicular lymphoma, grade 1, in 1996. Although initial chemotherapy was successful, he developed several recurrences of lymphoma over the following years. In May 2004, he presented with a discrete, single, massive parieto-occipital lobe brain lesion. The mass failed to regress with empiric cranial external beam radiotherapy. Because of suspicion of an unusual infection, the lesion was surgically excised in its entirety. The mass proved to be an aggressive diffuse large B-cell lymphoma, transformed from his previous follicular cell lymphoma, with retention of strong Bcl-2 and Bcl-6 immunoreactivity. CONCLUSIONS: Parenchymal brain involvement, as opposed to dural or leptomeningeal, is a relatively uncommon pattern of spread to the CNS for systemic lymphomas. More significantly, follicular lymphomas are one of the least frequent types of indolent lymphomas to develop clinically apparent, secondary CNS spread. The presentation of an indolent follicular lymphoma with transformation to an aggressive diffuse large B-cell lymphoma within the brain parenchyma is rare. Its manifestation as a massive, singular lesion is unique and prompted diagnostic confusion.     

Microfilarial involvement of the neck region: A case report and review of literature

IntroductionFilariasis is caused by parasitic infections, most commonly Brugia malayi and Wuchereria bancrofti. India is one of the endemic countries for such disease. The usually involved organs are lower limbs and external genitalia. Neck region is rarely involved and only a few cases have been reported in literature.Case reportWe reported such a case where filarial involvement has involved the neck region and reached up to the level of upper cervical vertebra. A huge neck swelling and torticollis were the main presenting features. Diagnosis was confirmed by MRI and live presence of microfilarial parasite in FNAC. The patient responded excellently to antihelminthic drugs. He has been under our regular follow-up and there has been no complication till date.DiscussionThe purpose of this case report was to highlight this rare presentation of microfilaria parasite.     

Clitoral enlargement caused by prepucial hemangioma: a case report]

A 4-year-old girl visited with the chief complaint of clitoral enlargement which was first pointed out when she was one year old. She has received excision of the femoral tumor, which was histologically an arteriovenous hemangioma, at 4 years old. The prepuce was darkly purplish and the clitoral neck was enlarged without enlargement of the clitoral glans. The external genitalia otherwise were normal and there were no virilizing signs. The mass was excised at the preoperative diagnosis of prepucial hemangioma. The mass was enclosed in the prepuce and adherent to its skin and there was no enlargement of the clitoris itself. The histopathological diagnosis was arteriovenous hemangioma.     

Melorheostosis: a case report and literature review

Melorheostosis is a rare form of cortical hyperostosis that resembles wax dripping down the side of a candle. This disease usually affects the long and short bones of an extremity. Literature review and a case report will be discussed with respect to incidence, clinical presentation, radiographic appearance, and treatment.     

Enchondroma: a case report and literature review

An unusual case of enchondroma located in the distal phalanx of the left third toe is presented with a review of literature. An enchondroma is a benign tumor centrally located in a bone. This osseous dysplasia is characterized by an excess of mature hypertrophic hyaline cartilage that has not resorbed or ossified in the normal fashion. Pathologic, clinical, and radiographic findings will be discussed.     

Ischemic intestinal involvement in a patient with Buerger disease: case report and literature review

A 42-year-old Japanese man who had undergone amputation of the left leg below the knee because of Buerger disease required emergency thrombectomy 7 months later. He complained of acute abdominal pain after thrombectomy. At aortography the distal superior mesenteric artery and its branches were not well visualized. Emergency laparotomy was performed because of suspected intestinal ischemia, and the terminal ileum and cecum and part of the ascending colon were resected. In total, the patient underwent laparotomy four times. Histopathologic findings revealed that the arteries and veins of the resected small intestine were occluded with organized thrombi. Inflammatory cell infiltration was recognized mainly in the intima. These findings are compatible with Buerger disease.     

Hepatic endometrioma: a case report and review of the literature: report of a case

Hepatic endometriosis has an extremely rare occurrence characterized by the presence of ectopic endometrium in the liver. A diagnosis of hepatic endometriosis is established after surgery. A 51-year-old multiparous female was referred to our unit for investigation of a liver tumor. The patient reported a 6-month history of epigastric pain and vomiting. She had undergone conservative hysterectomy for uterine leiomyomas several years earlier. The results of liver function tests and the levels of tumor markers (CA 19.9, CEA, CA125, αFP) were normal. Radiological imaging (USS, CT and MRI) suggested the presence of liver cystadenoma, liver cystadenocarcinoma or cystic metastasis of the liver in the left liver lobe extending to the diaphragm with left hepatic vein compression. Laparotomy was performed. The intraoperative frozen sections suggested a diagnosis of endometriosis. Anatomical resection was performed, including left lobectomy with diaphragm resection. The final histology confirmed the presence of hepatic endometrioma without malignant transformation. Fourteen cases of hepatic endometrioma have been described in the medical literature. We herein report the 15th case. Making a preoperative diagnosis of hepatic endometriosis is very difficult, despite conducting a complete investigation, in the absence of clinical and radiological characteristics. The diagnosis is made according to a histological examination of the whole surgical sample.     

Polyorchidism: a case report and review of the literature

Polyorchidism is an uncommon congenital defect. In fact, less than one hundred cases have been reported in the medical literature worldwide. This problem should be considered in the differential diagnosis of all scrotal masses. It is associated with multiple other urologic entities including malignancy. It is for this reason that it is important to be familiar with potential diagnostic and therapeutic strategies. This diagnosis is usually documented by imaging, specifically doppler ultrasound (US) and magnetic resonance imaging (MRI). The risk of malignancy approaches 6% in Polyorchidism and therefore several authors have advocated surgery as the most appropriate therapeutic approach. However, recent reports suggest that asymptomatic patients with normal imaging could be observed. We present a 14 year old boy with polyorchidism which was diagnosed clinically and confirmed with imaging. His treatment and a review of the literature are presented as well.     

Meningioangiomatosis: a case report and review of the literature

A case of cerebral meningioangiomatosis in an adult man without any stigmata of neurofibromatosis is reported. A 22 year-old man with no previous neurological history, presented with adversive seizures; clinical examination and electro-encephalogram were normal. CT showed nodular calcified masses in the left frontal lobe and in the left posterior parietal area. A craniotomy was performed and the frontal lesion was excised. Histological examination showed a predominantly intracerebral tumour involving both grey and white matter, with a complex picture of variable cellularity, dense calcification and prominent perivascular arrangements of reticulin-rich spindle cell fascicles with palisade formation widely involving the brain tissue. Immunohistochemistry for S100 protein varied between different parts of the tumour; there was focal positivity for S100 protein and smooth muscle actin in the lesion with no reaction for GFAP or EMA. Immunostaining for Factor 8-related antigen highlighted the perivascular arrangement of lesional cells and demonstrated an increase in the number of small vessels in other areas. Electron microscopy of the main mass showed elongated spindle cells with formation of pericellular basal lamina. The literature on meningioangiomatosis was reviewed. The evidence for meningeal, perivascular neural plexus or pericyte origin does not appear to be well founded. The present case further illustrates the difficulty in identifying an exact histogenetic cell and probably reflects an origin from a primitive perivascular mesenchymal cell.     

Polyorchidism: a case report and review of the literature

Polyorchidism is defined as the presence of two or more testes. It is a rare anomaly with approximately 77 cases reported in the literature. Polyorchidism is frequently associated with additional urological pathologies such as undescended testis, inguinal hernia, testicular torsion, hydrocoele, malignancy and infertility. Differential diagnosis includes spermatocoele, hydrocoele, epididymal cysts or aberrant epididymis. We report on an interesting case of polyorchidism in a 15-year-old man diagnosed on ultrasound and we review the literature and current management of polyorchidism.